What is Ravicti?
Ravicti (glycerol phenylbutyrate) binds with other substances in the liver and kidneys to help eliminate nitrogen from the body. Excess nitrogen can cause hyperammonemia, a build-up of ammonia in the blood. Ammonia is very toxic when it circulates in blood and tissues and can cause permanent brain damage, coma, or death.
Ravicti helps prevent a build-up of ammonia in the blood in people with urea cycle disorder. This medicine is used in adults, children and infants. Ravicti will not treat hyperammonemia.
Ravicti is usually given together with a low-protein diet and sometimes dietary supplements.
Ravicti can affect your nervous system. Call your doctor right away if you have: confusion, headaches, memory problems, hearing problems, vomiting, an altered sense of taste, numbness or tingling in your hands or feet, or if you feel unusually sleepy or light-headed.
Before taking this medicine
You should not use Ravicti if you are allergic to glycerol phenylbutyrate.
Do not give this medicine to a child younger than 2 years old without medical advice.
To make sure Ravicti is safe for you, tell your doctor if you have:
- liver or kidney disease;
- a pancreas disorder;
- stomach or intestinal disorder,
- a condition called NAGS (N-acetylglutamate synthase) deficiency; or
- if you use other medicines.
It is not known whether this medicine will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.
If you become pregnant, your name may be listed on a pregnancy registry. This is to track the outcome of the pregnancy and to evaluate any effects of Ravicti on the baby.
It is not known whether glycerol phenylbutyrate passes into breast milk or if it could harm a nursing baby. You should not breast-feed while using Ravicti.
How should I take Ravicti?
Use Ravicti exactly as prescribed by your doctor. Follow all directions on your prescription label. Your doctor may occasionally change your dose. Do not take this medicine in larger or smaller amounts or for longer than recommended.
Ravicti is usually taken 3 times per day. Take with food or infant formula.
Measure liquid medicine with the dosing syringe provided, or with a special dose-measuring spoon or medicine cup. If you do not have a dose-measuring device, ask your pharmacist for one.
If you are unable to swallow, you may take Ravicti through a nasogastric (NG) or gastric feeding tube as follows: After measuring the correct dose in the oral syringe, attach it to the feeding tube and push the plunger down to empty the syringe into the tube. Then flush the tube with 2 teaspoons of water and allow it to drain. Follow this with 2 more teaspoons of water to wash the contents down.
If you switch from sodium phenylbutyrate to glycerol phenylbutyrate, your dose will not be the same. Follow your doctor’s dosing instructions very carefully.
Ravicti is only part of a complete program of treatment that may also include a special diet and other medications. It is very important to follow the diet plan created for you by your doctor or nutrition counselor. You should become very familiar with the list of foods you should eat or avoid to help control your condition.
To be sure Ravicti is helping your condition and is not causing harmful effects, your blood will need to be tested often. A buildup of ammonia in the blood can quickly cause brain injury or death. Do not miss any follow up visits to your doctor for blood tests. Every person with a urea cycle disorder should remain under the care of a doctor.